Title : Sodium channelopathy with an overlap of brugada syndrome, paroxysmal atrial fibrillation, and progressive cardiac conduction system dysfunction
Abstract:
Background: Brugada syndrome is a rare diagnosis with a prevalence of 3 to 5 per 10,000, accounting for 4% of all sudden cardiac deaths. It can present with ventricular tachyarrhythmia and sudden cardiac deaths in structurally normal hearts, with an autosomal dominant pattern of inheritance. To diagnose it an ECG showing Brugada type 1 pattern with other clinical features is required. Different genes have been associated with disease, the commonest being voltage-gated sodium channel alpha type V gene (SCN5A).(1)
Case summary: We present a 21-year-old man who had 2 admissions from a local prison following a successfully resuscitated cardiac arrest. AED rhythm strip confirmed an underlying VF. The diagnosis of Brugada was based on both the ECG findings and the presentation of cardiac arrest. His ECG revealed coved ST changes in V1-2 and J waves at the inferolateral QRS complexes suggestive of Brugada pattern. Overlap of progressive cardiac conduction system dysfunction on top of the significant sodium channelopathy was due to the finding of prolonged first-degree AV block with paroxysmal atrial fibrillation. A Cardiac MRI showed a structurally normal heart. Isoprenaline infusion was intitiated and the amiodarone and beta-blocker therapy were stopped. Then transitioned to oral Quinidine sulphate (600mg bd) therapy with excellent effect. Inferolateral J waves disappeared and Quinidine-induced QT prolongation remained manageable. He displayed evidence of AV conduction disease with PR prolongation. A dual-chamber ICD was implanted for secondary prevention of arrhythmic death. He was then discharged back to his home, having completed his term in prison. Home monitor did not transmit any issues with his ICD, and his ICD checks were satisfactory. ICC clinic follow-up happened four months post-discharge, patient had no further events and remained asymptomatic. His ECG showed considerable improvement, PR interval was down to 200ms with a hint of type 1 Brugada-pattern in lead V1.
Conclusion: Reaching to a correct diagnosis and consulting the appropriate experts led to best possible outcome for the patient by having an Implantable Cardioverter Defibrillator (ICD) implanted, therefore preventing sudden cardiac deaths.
